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目的:分析胆道闭锁(biliary atresia，BA)患儿Kasai术后肝内胆管囊性扩张(intrahepatic biliary cysts，IBC)的形成特点及病理学表现，探索其与预后之间的关系。方法:选取2017年1月至2018年12月天津市儿童医院Kasai术后行肝移植手术的BA患儿179例(男75例，女104例)，收集患儿的临床资料和影像学资料，用以判断是否存在IBC，其中36例CT检查结果提示存在肝内胆管扩张，作为IBC(＋)组，143例未检测到肝内胆管扩张，作为IBC(-)组，比较两组的自体肝生存状况及肝功能指标。同时收集到上述患儿中50例患儿的肝移植时的病肝组织，其中IBC(＋)组18例，IBC(-)组32例，进行HE染色，观察肝脏纤维化程度、胆管增生程度、淤胆以及胆管板畸形的发生情况。结果:①IBC(＋)组女患儿有27例，占75.0%(27/36)，IBC(-)组女患儿有77例，占53.8%(77/143)，两组中女患儿占比的差异具有统计学意义(n P＝0.021)，两组患儿在行Kasai术时的日龄及术后胆管炎的发生率的比较中，差异无统计学意义(n P>0.05)；②IBC(＋)组患儿的中位生存期为11.5个月，明显高于IBC(-)组患儿的9.0个月。Log-Rank检测发现，IBC(＋)组术后自体肝生存状况优于IBC(-)组患儿，且差异具有统计学意义(n P＝0.038)；③肝功能的比较结果发现IBC(＋)组除胆汁酸(total bile acids，TBA)外，均低于IBC(-)组患儿，其中总胆红素(total bilirubin，TBI)、结合胆红素(direct bilirubin，DBI)水平在两组的差异具有统计学意义(n P0.05). Furthermore, median time of liver failure was longer in IBC (+ ) group than that in IBC (-) group (11.5 vs 9.0 months). Log-Rank test revealed that IBC (+ ) group was better than IBC (-) group and the difference was statistically significant (n P=0.038). Comparing liver function of two groups, except for TBA, other parameters of IBC (+ ) group were lower than those of IBC (-) group and inter-group differences in TB/DB level were statistically significant (n P<0.05). Degree of liver fibrosis, bile duct proliferation and bile duct plate deformity were slightly lower in IBC (+ ) group than those in IBC (-) group and the difference was not statistically significant. However, degree of portal bile plug was significantly lower in IBC(+ ) group than that in IBC(-) group and the difference was statistically significant (n P=0.013).n Conclusions:Intrahepatic biliary cyst after Kasai procedure may be a positive manifestation of liver compensation in BA children. In a short term, it will not increase cholestasis. On the contrary, IBC reduces cholestasis and liver function and extends the survival time of native liver.